Fig. 1.

The AI-family pedigree. The two hearing impaired brothers (AI-010 and AI-011) are homozygous for the c.292C>T(p.Arg98*) nonsense variant. Pure tone average (PTA) of right (R) and left (L) ears is represented in decibel hearing level (dB HL) for each family member. The older proband shows signs of prolonged vestibular impairment. All other family members, including one older sister (AI-000) and one older brother (AI-001), as well as both parents (AI-100 and AI-101), have normal hearing. All of them, except the older sister (AI-000), are carrying a heterozygous c.292C>T(p.Arg98*) variant